Hirschsprung's disease is a congenital disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation. The lack of ganglion cells, proven by Orvar Swenson to be the cause of the disease, disables the muscular peristalsis needed to move stool through the colon, thus creating a blockage. One in five thousand children suffer from Hirschsprung's. Four times as many males get this disease than females. Hirschsprung's develops in the fetus during the early stages of pregnancy. Typical symptoms for infants include not having their first bowel movement (meconium) within 48 hours of birth, and repeated vomiting. Some infants may have a swollen abdomen. Two thirds of the cases of Hirschsprung's are diagnosed within three months of the birth. Occasionally symptoms do not appear until early adulthood. A barium enema is the mainstay of diagnosis of Hirschsprung's, though a rectal biopsy showing the lack of ganglion cells is the only certain method of diagnosis.
The usual treatment is “pull-through” surgery where the portion of the colon that does have nerve cells is pulled through and sewn over the part that lacks nerve cells (National Digestive Diseases Information Clearinghouse). For a long time, Hirschsprung's was considered a multi-factorial disorder, where a combination of nature and nurture were considered to be the cause (Madsen 19). However, in August of 1993, two articles by independent groups said that Hirschsprung's disease could be mapped to a stretch of chromosome 10. This research also suggested that a single gene was responsible for the disorder. However, the researchers were unable to isolate the single gene that they thought caused Hirschsprung's.
There used to be two steps typically used to treat Hirschspring's. The first stage used to be a colostomy. When a colostomy is performed, the large intestine is cut and an opening is made through the abdomen. This allows bowel contents to be discharged into a bag. Later, when the child's weight, age, and condition is right, a pull-through procedure is performed. The pull-through procedure repairs the colon by connecting the functioning portion of the bowel to the anus. The pull through procedure is the typical method for treating Hirschsprung's in younger patients. Swenson devised the original procedure, but the pull-through surgery has been modified many times. Pull-through procedures used to require a colostomy, but with increased awareness among doctors and parents about the symptoms of Hirschsprung's and with early diagnosis, doctors can keep the colon clean and perform the pull-through procedure without a colostomy. In general, 85 percent of patients that have the pull-through surgery live normal lives afterwards. The other 15 percent have to take a laxative for the rest of their lives.
Frequently these laxatives are administered in a suppository form, requiring the patient to remain in a specific, and often uncomfortable, position for an extended period of time. Thus, there are present and continuing needs for new and improved body support for medical applications, especially those with Hirschsprung's.